摘要:

After exposure of C57BL6 × DBA/2 mice to benzene in air their number of bone marrow fibroblastoid precursor cells, CFU-F, was determined. The CFU-F exhibited an increasing plating efficiency, giving rise to a larger number of colonies and to colonies of greater size. This effect was dose dependent. When the mice were exposed for 16 weeks and were then allowed to rest, their CFU-F plating efficiency returned to normal within 6 weeks, but then increased again. Hematopoietic stem cells, such as CFU-S and CFU-C exhibited a dose-dependent depression. The in vitro exposure of bone marrow cells to benzene metabolites resulted in a dose-dependent depression of CFU-F numbers

ISSN:0001-5792    

DOI:10.1159/000204828

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

We investigated the molecular bases for a mild phenotype by α-, β- and γ-globin gene analyses in 22 patients with transfusion-independent thalassemia intermedia (15) or a late-presenting form of thalassemia major (7) originating from Puglia, a region of southern Italy. Twenty-two patients with thalassemia major served as controls. The β+ IVS-I nt 6 of the β-globin gene and the C→T substitution at position -158 5’ of the Gγ-globin gene were detected more frequently in patients with thalassemia intermedia or late-presenting thalassemia major considered together as compared to those affected by typical transfusion-dependent thalassemia major. Three of 15 patients with thalassemia intermedia had the triple α-globin gene arrangement in the heterozygous (2) or homozygous state (1) in association with heterozygous β°-thalassemia. From these results, we may conclude that the inheritance of a mild β-thalassemia allele such as the β+ IVS-I nt 6 mutation, in the homozygous or heterozygous state, the coinheritance with homozygous β°-thalassemia of the -158 (C→T) Gγ gene promoter mutation and the presence of heterozygous β-thalassemia/triple α-globin gene arrangement are the most common reasons accounting for the development of attenuated forms of β

ISSN:0001-5792    

DOI:10.1159/000204829

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

We report the nucleotide (nt) substitutions of four unrelated glucose-6-phosphate dehydrogenase (G6PD)-deficient males. Only the mutation of G6PD Wayne was unique. It was a nt 769 C→G substitution causing a deduced substitution of glycine for arginine at amino acid 257. This mutation is in a region in which G6PD mutations have previously been associated with chronic hemolytic anemia. The mutation of G6PD Jammu and G6PD Viangchan were identical: a G→A mutation at nucleotide 871, predicting a Val→Met substitution at amino acid 291. However, these two variants differ with respect to the 1311 polymorphism, suggesting that they may have arisen independently. Enzyme from a child with chronic hemolytic anemia, designated G6PD ‘LeJeune’, proved to be due to a G→T substitution at nt 637, a change identical with that in 3 unrelated patients who had been reported previously as having G6PD Gastonia, Minnesota and Marion. These findings support the suggestion that both polymorphic and sporadic G6PD deficiency mutations in unrelated persons with G6PD deficiency are often the same, even when thought to be distinct on the basis of biochemical chara

ISSN:0001-5792    

DOI:10.1159/000204830

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

We investigated the alteration of CuZn-superoxide dismutase during erythroid and myeloid differentiation in order to elucidate its physiological significance in different types of cells. We measured enzyme activity and mRNA levels of superoxide dismutase in the process of differentiation to erythroid cells or myeloid cells. When human leukemia K562 cells are incubated in the presence of 80 μM hemin, benzidine-positive cells appear on day 1 and 80% of the cells become positive on day 5. During hemin-induced erythroid differentiation, CuZn-superoxide dismutase activity increases 3.5-fold of the initial value and mRNA for CuZn-superoxide dismutase increases prior to the activity to the same extent. On the other hand, when human pro-myelocytic leukemia HL-60 cells are incubated in the presence of 1.3% dimethyl sulfoxide, nitroblue tetrazoli-um-positive cells reach approximately 90% on day 5. During dimethyl sulfoxide-induced myeloid differentiation, the activity of CuZn-superoxide dismutase decreases below 15% of the initial value on day 5 and mRNA for CuZn-superoxide dismutase decreases as well. The results indicate that the synthesis of superoxide dismutase is linked with either the erythroid or myeloid differentiation program

ISSN:0001-5792    

DOI:10.1159/000204831

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

The ability of peripheral-blood hematopoietic progenitor cells from AIDS patients and normal controls to respond to erythropoietin (Epo) was assessed for burst-forming units-erythroid (BFU-E). BFU-E colony formation from AIDS patients’ peripheral blood responded to a wide range of Epo concentrations (0.5–4 U) in a similar manner as erythroid progenitors obtained from normal peripheral blood. The optimum dose response of BFU-E to Epo was 2 U which resulted in generation of 71 ± 4 BFU-E in AIDS patients (n = 10), as compared to 77 ± 5 BFU-E in normal donors (n = 3). The optimum concentration range of hemin enhancement of erythroid progenitor BFU-E was 10–50 μM. In all instances, Epo was essential for BFU-E growth. Inclusion of hemin at a concentration of 10 μM in AIDS patients’ peripheral-blood erythroid progenitor cells resulted in enhancement of BFU-E by 136–215%. Similarly, inclusion of hemin (10–100 μM) in normal bone marrow erythroid progenitor cell cultures resulted in enhancement of BFU-E. Inclusion of an equivalent amount of iron or tin protoporphyrin to progenitors cells from AIDS patients’ peripheral blood had no effect on the number of colonies observed. On the other hand, inclusion of another heme analogue, zinc protoporphyrin, in AIDS or normal cultures resulted in a 50% suppression of BFU-E colony formation. These results demonstrate that peripheral-blood mononuclear cells from AIDS patients retain the capacity to generate erythroid precursors such as BFU-E in the presence of Epo, and that hemin has a specific enhancement effect on growth of BFU-E colony formation obtained from peripheral blood or

ISSN:0001-5792    

DOI:10.1159/000204832

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

A Spanish family was found to have the coexistence of a hereditary haemolytic syndrome associated with excessively leaky RBC membrane to sodium (Na+) and potassium (K+) cations and a partial coagulation factor VII deficiency. Haemolysis was mild in the propositus and the RBC membrane leak included a marked increase in passive permeability to Na+ and K+. This was associated with an increase in active Na+, K+-pump activity and in the ouabain-resistant fluxes: Na+, K+-cotransport and Na+, Li+-countertransport. Factor VII deficiency was of 50% and no clinical expression of the coagulation deficiency was observed. The family study revealed slightly abnormal RBC membrane cationic fluxes only in the father and decreased coagulation factor VII activity of 67% in the mother. Both parents were clinically and haematologically normal. It is suggested that the propositus has inherited the abnormal gene for leaky RBC syndrome from the father and the partial coagulation factor VII deficiency from the mother.

ISSN:0001-5792    

DOI:10.1159/000204833

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

A case of Evans’ syndrome which was refractory to conventional and high dose steroid treatment and to splenectomy was treated with cyclosporin, beginning with the dose of 10 mg/kg/day and then gradually tapering to 4 mg/kg/day. The patient experienced excellent benefit from cyclosporin; his haematological parameters were completely normal at the 12th month of therapy, without any side effect of the dru

ISSN:0001-5792    

DOI:10.1159/000204834

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

A 47-year-old woman with progressive systemic sclerosis developed disseminated intravascular coagulation in the course of her terminal illness. She also had complicating necrotizing angiitis and generalized lymphadenopathy. The likely relationship between disseminated intravascular coagulation and vasculitis is discussed.

ISSN:0001-5792    

DOI:10.1159/000204835

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

The immunohistochemical localization of the peptide γ-melanocyte-stimulating hormone (γ-MSH) within human polymorphonuclear leucocytes of blood from eosinophilic patients is described. The γ-MSH immunoreactivity was observed only in neutrophilic granulocytes leaving all other cell types immuno-negati

ISSN:0001-5792    

DOI:10.1159/000204836

出版商:S. Karger AG    

年代:1991

数据来源: Karger

摘要:

A case of chronic lymphocytic leukemia is described in which peripheral blood and bone marrow films showed lymphocytes arranged in aggregates resembling epithelial cell clusters. Due to this morphological conformation, automatic hemogram screening was not able to detect the presence of chronic lymphocytic leukemia. Correct interpretation of a typical histogram pattern led to the final diagnosis.

ISSN:0001-5792    

DOI:10.1159/000204837

出版商:S. Karger AG    

年代:1991

数据来源: Karger